MuSK抗体阳性重症肌无力研究进展  

Research Progress of Myasthenia Gravis with Antibodies to MuSK

作　　者：李斌[1] 刘曼[1] 宋晓文[1] 庄伟 董晓梦[1] 陈金波[1] LI Bin;LIU Man;SONG Xiaowen;ZHUANG Wei;DONG Xiaomeng;CHEN Jinbo(Binzhou Medical University Hospital,Binzhou 256600,China;不详)

机构地区：[1]滨州医学院附属医院,山东滨州256600

出　　处：《中国医学创新》2021年第15期177-180,共4页Medical Innovation of China

基　　金：国家自然科学基金青年科学基金项目(81701192);山东省医药卫生科技发展计划项目(2019WS322);滨州医学院科技计划项目(BY2020KJ17)。

摘　　要：重症肌无力(myasthenia gravis,MG)是一种自身抗体介导累及神经肌肉接头(NMJ)突触后膜的自身免疫性疾病,具有骨骼肌易疲劳性、症状波动性等特点,抗肌肉特异性受体酪氨酸激酶(MuSK)抗体阳性重症肌无力(MMG)患者占MG的5%~10%。与抗乙酰胆碱受体(AChR)抗体阳性的MG相比,具有发病时间早、病情重、疗效不佳、病情易进展等特点,其中急性发作和延髓肌受累是最常见、最重要的临床特点。目前,国内外对关于MuSK抗体相关发病机制研究缺乏,其发参与的信号通路亦不明确。本文就MuSK抗体介导的MG发生发展过程进行综述,以期对未来该病的诊治及预防提供理论依据。Myasthenia gravis(MG)is an autoimmune disease with autoantibody mediated involvement of the postsynaptic membrane of the neuromuscular junction(NMJ),which is characterized by skeletal muscle fatigue and symptom fluctuation,etc.The patients with myasthenia gravis(MMG)positive anti-muscle-specific receptor tyrosine kinase(MuSK)antibody account for 5%-10%of MG.Compared with the anti-acetylcholine receptor(AChR)antibody positive MG,it has the characteristics of early onset,severe disease,poor efficacy and easy progression,among which acute attack and medulla oblongata muscle involvement are the most common and important clinical characteristics.At present,there is a lack of research on the pathogenesis related to MuSK antibody at home and abroad,and the signaling pathway involved in MuSK antibody is not clear.In this paper,the occurrence and development of MuSK-mediated MG were reviewed in order to provide theoretical basis for the diagnosis,treatment and prevention of the disease in the future.

关 键 词：重症肌无力 肌肉特异性受体酪氨酸激酶抗体 抗乙酰胆碱受体 发病机制 神经-肌肉接头 

分 类 号：R746.1[医药卫生—神经病学与精神病学]